BACKGROUND: Pulmonary atresia with ventricular septal defect without major aortopulmonary collateral arteries (MAPCAs) is an uncommon form of congenital heart disease. As more patients with congenital heart disease live to adulthood, the objective of this article was to review the long-term results of this specific population.
METHODS: A review of the PubMed database was performed using pertinent key words (pulmonary atresia, tetralogy of Fallot, conduit, right ventricle-pulmonary artery) concentrating on studies from 1990-present and published in English. Most studies of pulmonary atresia-ventricular septal defect patients included those with and without MAPCAs. Analysis included examination of the entire cohort, consideration of the proportion of patients with MAPCAs, and any subgroup analysis of the patients without MAPCAs.
RESULTS: Survival is approximately 80% at ten years and is improved with complete repair and larger pulmonary arteries. Some studies have found genetic syndromes and extracardiac anomalies to impact survival, while others have not. Incomplete repair has been shown to be associated with worse survival. Independent of initial management strategy, patients with pulmonary atresia and ventricular septal defects without MAPCAs require repeat intervention on the right ventricular outflow tract. Hypoplastic pulmonary arteries have been shown to be a risk factor for reintervention, and decreased conduit durability has been shown with younger age at implantation of conduit.
CONCLUSIONS: Long-term outcomes have improved for patients with pulmonary atresia-ventricular septal defect without MAPCAs, with complete repair and adequate pulmonary arteries favorable for survival. Long-term outcomes include reinterventions, both catheter-based and surgical, predominantly on the right ventricular outflow tract.

Blood-filled cysts of the heart valves are frequently reported at postpartum autopsies of infants. They are seen as round nodules mostly in the paediatric age group in infants less than 2 months of age and disappear spontaneously within 6 months of life. We report a unique case of an 11-month-old girl who presented at a tertiary healthcare hospital in 2022 with a blood-filled cyst on the posterior leaflet of the pulmonary valve that was successfully treated. This case report highlights the characteristics and course of a paediatric patient with blood-filled cysts. Further studies are yet needed to better understand the diagnostic approaches to blood-filled cysts as well as treatment modalities to fill the gap in clinical settings.

Pulmonary arterial sarcomas (PAS) are rare aggressive tumours occurring mainly in the pulmonary trunk. We report a case of PAS involving the pulmonary trunk wall and valve, with uniform wall thickening which represents an atypical imaging manifestation of this tumour. A 63-year-old male presented with vague respiratory symptoms with rapid progression. CTPA showed low density filling defects in both pulmonary arteries and PET scan showed increased uptake in the pulmonary trunk, which along with raised ESR suggested Pulmonary Vasculitis. Echo imaging showed Right ventricular hypertrophy and pulmonary stenosis. Response to steroid therapy was minimal and his symptoms worsened. A referral for second opinion was made and he was diagnosed with PAS. He underwent Pulmonary thromboendarterectomy with Pulmonary valve replacement. Post-operative histopathology confirmed the diagnosis. PAS is rare and frequently misdiagnosed. Surgical resection is not curative, but together with chemotherapy can prolong survival.

Marfan syndrome (MFS) is a progressive connective tissue disease with a broad range of clinical manifestations. We sought to establish the spectrum of structural valvular abnormalities as cardiovascular involvement has been identified as the most life-threatening aspect of the syndrome. This was a systematic review with a meta-analysis of studies indexed in Medline from the inception of the database to November 7, 2022. Using the random-effects model, separate Forest and Galbraith plots were generated for each valvular abnormality assessed. Heterogeneity was assessed using the I2 statistics whilst funnel plots and Egger\'s test were used to assess for publication bias. From a total of 35 studies, a random-effects meta-analysis approximated the pooled summary estimates for the prevalence of cardiac valve abnormalities as mitral valve prolapse 65% (95% CI: 57%-73%); mitral valve regurgitation 40% (95% CI: 29%-51%); aortic valve regurgitation 40% (95% CI: 28%-53%); tricuspid valve prolapse 35% (95% CI: 15%-55%); and tricuspid valve regurgitation 43% (95% CI: 8%-78%). Only one study reported on the involvement of the pulmonary valve (pulmonary valve prolapse was estimated at 5.3% (95% CI: 1.9%-11.1%) in a cohort of 114 patients with MFS). We believe this study provides a description of the structural valvular disease spectrum and may help inform providers and patients in understanding the clinical history of MFS in the current treatment era with its increased life expectancy.

Background: The Ross-Konno procedure is a technically demanding surgical option to treat multilevel left ventricular outflow tract obstruction. Methods: A systematic review with pooled analyses was conducted according to PRISMA criteria on studies published between January 2000 and May 2022 that assessed outcomes following the Ross-Konno intervention in children. Individual patient data were extracted from published Kaplan-Meier curves using digitalization software. Overall survival and freedom from reintervention were assessed by time-to-event approaches. Determinants of one-year survival were investigated by meta-regression analyses. Results: Ten studies with a total population of 274 patients were included. The overall pooled early (≤30 days) survival rate was 86.9% (95% CI [87.6%-78.4%]). Five-year survival rates in patients without and with (N = 50 [18.2%] of 274 total patients) concomitant mitral valve surgery were 82.5% (95% CI [87.6%-77.4%]) versus 56.1% (95% CI [74.1%-38.1%]), hazard ratio 2.67, 95% CI (1.44-4.93), P < .0001. Five- and ten-year freedom from pulmonary autograft reoperation rates were 93.5% and 90.9%, respectively. Five- and ten-year freedom from right ventricular outflow tract reoperation rates were 74.3% and 57.3%, respectively. By meta-regression analysis, resection of endocardial fibroelastosis (N = 32 [11.7%] of 274 total patients) was associated with superior one-year survival (P = .027). Conclusion: The Ross-Konno procedure is associated with substantial early mortality and gradual attrition thereafter. Mortality is higher in patients with concomitant mitral valve surgery. Resection of endocardial fibroelastosis is associated with superior survival. Right ventricular outflow tract reinterventions are common.

There is conclusive evidence for relief of pulmonary valve obstruction immediately after balloon pulmonary valvuloplasty (BPV) and at follow-up. Development of infundibular obstruction is seen in more severe PS cases and in older subjects. Reappearance of PS was observed in approximately 10 % of patients following BPV. The reasons for recurrence were found to be balloon/annulus ratio less than 1.2 and immediate post-BPV pulmonary valve peak gradients greater than 30 mmHg. Recurrent stenosis is successfully addressed by repeating BPV with lager balloons than used initially. Long-term results revealed continue relief of obstruction, but with development of pulmonary insufficiency, some patients requiring replacement of the pulmonary valve. It was concluded that BPV is the treatment of choice in the management valvar PS and that balloon/annuls ratio used for BPV should be lowered to 1.2 to 1.25. It was also suggested that strategies should be developed to prevent/reduce pulmonary insufficiency at long-term follow-up.

UNASSIGNED: The optimal aortic valve substitute for non-elderly adults remains controversial. Recently, considerable data on the Ross procedure have accumulated. This study aimed to analyze long-term outcomes following the Ross procedure from the current literature using a meta-analysis of time-to-event outcomes.
UNASSIGNED: A literature search was performed with MEDLINE, EMBASE, Cochrane Library, Web of Science, and Google Scholar through June 2022; studies reporting clinical outcomes of the Ross procedure beyond 20 years were included for analysis. The outcomes of interest were late survival and freedom from surgical or percutaneous reintervention of the autograft or right ventricular outflow tract (RVOT).
UNASSIGNED: Six studies, including 4,910 patients (3,601 males), were identified and analyzed. Survival rate at 5, 10, 15, and 20 years was 99.9%±0.1%, 97.6%±0.5%, 94.3%±0.9%, and 87.4%±1.9%. Freedom from autograft reintervention at 5, 10, 15, and 20 years was 97.7%±0.5%, 95.3%±0.7%, 91.4%±1.2%, 84.8%±2.5%. Freedom from RVOT reintervention was 99.0%±0.3%, 99.0%±0.3%, 97.5%±0.7%, 93.3%±1.8%. Freedom from any valve reintervention (either autograft or RVOT) at 5, 10, 15, and 20 years was 95.8%±0.6%, 92.6%±0.9%, 88.5%±1.2%, 80.8%±2.5%.
UNASSIGNED: This meta-analysis demonstrated that the Ross procedure was confirmed to provide excellent survival despite the need for reintervention of autograft or RVOT in approximately 20% of patients at 20 years.

BACKGROUND: Several factors increase the risk of right-sided endocarditis. The tricuspid valve is usually involved in right-sided endocarditis cases. Infective endocarditis of the pulmonic valve is rare, and few cases of pulmonic valve endocarditis were reported previously.
METHODS: Here we describe a case of a 81-year-old Middle Eastern male patient, admitted to our hospital three times in a period of 2 months for fever and cough. He had Streptococcus oralis bacteremia with vegetation that was on the pulmonic valve. We diagnosed him with pulmonic valve endocarditis, and he was treated successfully with intravenous antibiotics.
CONCLUSIONS: It is important to keep high suspicion for isolated pulmonic valve endocarditis in patients with respiratory symptoms. Adequate dental care is important in patients with risk factors for infective endocarditis.

BACKGROUND: Cardiac myxoma is the most common type of primary cardiac tumor, with the majority located in the atrial wall. The tumor is attached to valvular structures in a few cases, of which the pulmonary valve is the least affected. Pulmonary valve myxoma may have different clinical manifestations from the more common cardiac myxomas because of its vital position. A misdiagnosis of these types of cardiac myxoma may be detrimental to the care and well-being of patients. Therefore, this systematic review aims to define the clinical characteristics of pulmonary valve myxoma and how this differs from a more common cardiac myxoma.
METHODS: Employed literature was obtained from PubMed, ScienceDirect, Scopus, Springer, and ProQuest without a publication year limit on August 23, 2022. The keyword was \"pulmonary valve myxoma.\" Inclusion criteria were as follows: (1) case report or series, (2) available individual patient data, and (3) myxoma that is attached to pulmonary valve structures with no evidence of metastasis. Non-English language or nonhuman subject studies were excluded. Johanna Briggs Institute checklists were used for the risk of bias assessment. Data are presented descriptively.
RESULTS: This review included 9 case reports from 2237 articles. All cases show a low risk of bias. Pulmonary valve myxoma is dominated by males (5:4), and the patient\'s median age is 57 years with a bimodal distribution in pediatric and geriatric populations. The clinical manifestation of pulmonary valve myxoma is often unspecified or asymptomatic. However, systolic murmur in the pulmonary valve area is heard in 67% of cases. Echocardiography remains the diagnostic modality of choice in the majority of cases. Tumor attached to the pulmonary cusps or annulus and extended to adjacent tissues in all cases. Therefore, valve replacement or adjacent tissue reconstructions are required in 77% of cases. The recurrence and mortality are considerably high, with 33% and 22% cases, respectively.
CONCLUSIONS: Pulmonary valve myxoma is more common in males with a bimodal age distribution, and its outcomes seem worse than usual cardiac myxomas. Increasing awareness of its clinical symptoms, early diagnosis, and complete myxoma resection before the presence of congestive heart failure symptoms are important in achieving excellent outcomes. A firm embolization blockade is needed to prevent myxoma recurrence.

This study aims to perform a meta-analysis of early and late outcomes of the Ross/Ross-Konno procedures in neonates/infants.
A meta-analysis was performed in accordance with PRISMA guidelines. We used Ovid versions of MEDLINE/PubMed for relevant studies and included those that reported Ross/Ross-Konno operations in neonates/infants and at least one of the predetermined clinical outcomes. I2 and double arcsine methods assessed the heterogeneity between pooled estimates. We used a random-effect model to account for heterogeneity with MetaXL. We calculated point estimates of a pooled estimates along with its 95% CI.
587 neonate/infant patients were included with median age of 87.5 days old. The follow-up range was five days to 23 years. Early mortality reported in 25 studies with pooled estimates of 18.3% (95% CI: 13.6%-23.5%). Estimates ranged from 0% to 50% with relatively substantial heterogeneity (P = .01, I2 = 48.6%). Late mortality reported in 22 studies with pooled incidence of 9.7% (95% CI: 5.9%-14.3%). Estimates ranged from 0% to 53% with relatively substantial heterogeneity (P = .01, I2 = 46.1%). Autograft reintervention reported in 18 studies with pooled estimate of 19.2% (95% CI: 7.3%-34.5%). Estimates ranged from 0% to 81.8% with high heterogeneity (P < .001, I2 = 90.5%). Right ventricle-to-pulmonary artery conduit reintervention reported in 16 studies with pooled estimates of 32.0% (95% CI: 20.9%-44.12%). Estimates ranged from 0% to 92.3% with high heterogeneity (P < .001, I2 = 75.9%).
The data suggest that the Ross/Ross-Konno procedure in neonates/infants still carries significant risk of early/late mortality and autograft/conduit reintervention. The high variability of results among centers confirms the need for surgical expertise and good patient selection. Prospective multicenter studies are warranted to investigate the rate of autograft reintervention and the impact on long-term survival in this specific population.